Anaesthetic management of Stiff Man syndrome

Abstract

Stiff Man syndrome or stiff-person syndrome is a rare autoimmune disorder. It is characterized by increased axial muscular tone and limb musculature, and painful spasms triggered by stimulus. The case is presented of a 44-year-old man with stiff-person syndrome undergoing an injection of botulinum toxin in the urethral sphincter under sedation. Before induction, all the surgical team were ready in order to minimise the anaesthetic time. The patient was monitored by continuous ECG, SpO2 and non-invasive blood pressure. He was induced with fractional dose of propofol 150 mg, fentanyl 50 μg and midazolam 1mg. Despite careful titration, the patient had an O2 saturation level of 90%,which was resolved by manual ventilation. There was no muscle rigidity or spasm during the operation. Post-operative recovery was uneventful and the patient was discharged 2 days later. A review of other cases is presented. The anaesthetic concern in patients with stiff-person syndrome is the interaction between the anaesthetic agents, the preoperative medication, and the GABA system. For a safe anaesthetic management, total intravenous anaesthesia is recommended instead of inhalation anaesthetics, as well as the close monitoring of the respiratory function and the application of the electrical nerve stimulator when neuromuscular blockers are used.

Keywords: Anaesthetic considerations; Anaesthetic management; Consideraciones anestésicas; Manejo anestésico; Stiff Man syndrome; Síndrome de la persona rígida.